Meconium Ileus

Meconium Ileus is usually a manifestation of cystic fibrosis. Meconium is normally stored in the infant's intestines until after birth, but sometimes it is expelled into the amniotic fluid prior to birth or during labor and delivery . Occasionally, however, meconium ileus may be seen in association with pancreatic atresia or stenosis of the pancreatic duct. Sometimes the meconium becomes thickened and congested in the ileum , a condition known as meconium ileus . Although air can flow past the meconium trapped in the baby's airways as the baby breathes in, the meconium becomes trapped in the airways when the baby breathes out. The condition also occurs in other individuals of other races. Meconium ileus results from a deficiency of trypsin and other digestive enzymes from the pancreas, as in cystic fibrosis (fibrocystic disease of the pancreas, mucoviscidosis).

Meconium Ileus is classified into two types:

(1) Simple meconium ileus: The distal small bowel (10-30 cm of distal ileum) is relatively small, measuring less than 2 cm in diameter and contains concretions of gray, inspissated meconium with the consistency of thick glue or putty. It is often beaklike in appearance, conforming to the shape of the contained pellets. Proximally, the mid-ileum is large, measuring up to 7 cm in diameter. It is greatly distended by a mass of extremely thick, tenacious, dark green or tarry meconium. The unused small colon (microcolon) contains a small amount of inspissated mucus or grayish meconium.

(2) Complicated meconium ileus: usually occurs during the prenatal period associated to volvulus, atresias, gangrene, perforation or peritonitis. A cystic mass or atresia of the bowel may occur. The degree of obstruction varies, may be cured in mild cases by rectal irrigations. Failure to pass meconium, abdominal distension and vomiting are seen in more severe cases.

These cases not withstanding, meconium ileus is usually taken to be synonymous with cystic fibrosis until proven otherwise, and meconium ileus is generally the earliest manifestation of the disease Meconium can be inhaled into the lungs when the baby gasps while still in the womb or during the initial gasping breaths after delivery. In meconium ileus, low or distal intestinal obstruction in results from the impaction of thick, tenacious meconium in the distal small bowel.

Cause of Meconium Ileus

Here are the list of the possible cause of Meconium Ileus:

• Genes are the chemical units in every cell that tell cells what functions they should perform and what substances they should manufacture in order to operate normally.
• Due to the significant variability in disease presentation, evidence supports the role of modifier genes.
• The most common mutation of the CFTR gene is a 3 base pair deletion that removes a phenylalanine residue at amino acid position 508 of CFTR. This is called the DF508 mutation.
• The regulation of these genes and processes may help explain the range of variability in similar genetic mutations.
• The management of such patients proved to be a challenge to clinicians because of the rarity of this condition.
• The CFTR gene carries instructions for the production of mucus in cells.

Symptoms of Meconium Ileus

Some sign and symptoms related to Meconium Ileus are as follows :

• One of the first symptoms of CF in young babies is meconium ileus (pronounced muh-KO-nee-um ILL-ee-us). Meconium ileus is characterized by a thick, sticky, dark stool.
• abdominal cramping
• abdominal distention
• Persisten trespiratory infections such as pneumonia
  Coughing or wheezing
  Pneumonia is a lung infection that can be caused by a number of microorganisms.
• The next step is often a Gastrografin enema (meglumine diatriazoate), to attempt to wash out the meconium. If this does not do the trick, then a surgical operation is required to remove the meconium.
• Loops of distended small bowel can sometimes be palpated through the abdominal wall and may feel characteristically doughy.
• The patient may not grow to normal size without the nutrients provided by food.

Treatment of Meconium Ileus

Therapy is either:

(1) Nonoperative- should be tried first. It consist of a careful gastrograffin enema after the baby is well-hydrated. Gastrograffin is a hyperosmolar aqueous solution of meglumine diatrizoate containing 0.1% polysorbate-80 (tween-80, a wetting agent) and 37% iodine. Its success is due to the high osmolarity (1700 mosm/liter) which draws fluid into the bowel and softens and loosens the meconium.

(2) Surgical therapy - that has included: ileostomy with irrigation, resection with anastomosis, and resection with ileostomy (Mikulicz, Bishop-Koop, and Santulli). Post-operative management includes: 10% acetylcysteine p.o., oral feedings (pregestimil), pancreatic enzyme replacement, and prophylactic pulmonary therapy. Long-term prognosis depends on the degree of severity and progression of cystic fibrosis pulmonary disease.

Prevention Tips

• The infant must show signs of uncomplicated MI and no clinical or radiologic evidence of complicating factors (eg, volvulus, gangrene, perforation, peritonitis, atresia of the small bowel).
• There is no cure for cystic fibrosis, but there are many types of treatment that can help patients live longer and more comfortable lives.
• A nasogastric tube is a tube inserted through the nose, down the throat, and into the stomach
• Contrast agent for "barium enema" remains in the system for a long time (many days); normally this would be passed in 24hr or so. This indicates that motility is reduced (even though normal bowel sounds might be present, and passage is clear).
• The enema must be performed under fluoroscopic control.
• In some cases, especially where there is a mechanical obstruction, surgery may be necessary.

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